Changing the Lives of Patients with

Myotubular and Centronuclear Myopathies (CNM)

Meet Prince

As she neared the end of her pregnancy, Oluronke eagerly awaited the birth of her third child. Everything seemed normal and routine, except for an elevated amount of amniotic fluid. Doctors were not concerned and told her there was no reason for her to worry. But when her son Prince was born, there was no cry, little movement, and he had visibly poor muscle tone. A tense team gathered immediately in the delivery room to resuscitate him. Finally, after receiving oxygen, he gave out a barely audible cry. As Oluronke looked back at this moment, she said, “This is how our journey with X-linked Myotubular Myopathy began.”

Despite his dramatic entrance into the world, doctors assured Oluronke that Prince was fine. But without a specific answer to explain his weakness, he was transferred from the smaller hospital near their home to the larger Duke University Hospital within 24 hours of his birth. For Oluronke, she took this need to transfer Prince as a sign that his condition was actually quite serious. She was right.

Weeks went by in the hospital, and the medical staff continued to be at a loss for answers. They focused on keeping Prince medically stable and safe, avoiding specific treatments since they did not know the root cause of his symptoms. Prince continued to receive oxygen regularly, struggled to feed and lost precious weight. And after two months, genetic testing finally confirmed a diagnosis.

Prince had X-linked myotubular myopathy (XLMTM), a rare genetic neuromuscular disorder characterized by muscle weakness that can range from mild to very profound and debilitating. XLMTM causes weakness in muscles that are involved in swallowing and breathing, and patients often have severe respiratory distress and require ventilation. A rare disorder affecting about 1 in 50,000 male births in the U.S., XLMTM can bring potentially life-threatening complications during infancy and early childhood.

Doctors prepared Oluronke and her husband for the worst. They told the family that Prince would require support for the rest of his life – if he even made it out of the hospital. “I was in such shock that I could not absorb what the doctors were saying,” explained Oluronke. “I did not know where to start, how to help my son. I had never been exposed to a child so medically compromised. It was a nightmare and I wanted just to wake up.”

While still in the hospital, Oluronke researched XLMTM on her own, reached out through social channels to other families, and worked with the hospital staff on how to best care for her medically fragile son. Prince received a tracheostomy, which Oluronke believes gave him new strength. He began to hold hands, to grab for things. With faith and family, she became determined to move past her fear and embrace the challenges ahead. After four months of hospitalization, Prince went home.

Prince reunited with his two older siblings for just the second time in their lives. They had questions but embraced their little brother with joy and unconditional love. Oluronke drew strength from their example. “If they can, I can.”

“I believe it is only by miracles that Prince is still alive. I have seen my child turn blue and turn gray right before my eyes. I have been there, I have done that. Now I live in the moment, I spend my time loving and thoroughly caring for my child.”– Oluronke, mom to Prince

Life has not been easy. Since Prince requires 24-hour care, Oluronke had to quit her job which has added financial stress to the family of five. They became siloed from the rest of the world since it was too hard to bring Prince outside of the home. “I was limited to the four corners of my house for a year,” said Oluronke. “The amount of equipment to keep him alive is daunting, and it was easier to keep him home and protected.” And just as the family became more confident in taking Prince outside and to see other people, the global Coronavirus pandemic has forced them stay confined long term, leaving the house only to attend medical appointments and occasionally to visit Oluronke’s mom.

Oluronke wanted to share their journey with XLMTM to bring awareness not only to the struggles of daily life, but to serve as a source of encouragement for others. “I was the person who was crying and depressed. The first few months were so rough,” Oluronke said. “But it got better. Our son brings so much joy to our family. He is happy, he loves books and music. Life is different, but there are no dull moments. He pushes us and we are more loving and happy because of it.”

“Prince does not have much strength and needs a great deal of support. But we do enjoy him for the miracle he is.” said Oluronke. She also adds that the family has hopes for Prince for the future but focuses on short term goals she believes are attainable for her son, like perhaps sitting up for a longer period of time. “My faith does not allow me to give up,” she said. “I will remain open and optimistic and will work to bring out the best in my son.” Today Prince makes sounds that Oluronke hopes will turn into words. “I hear him, and I want others to hear his voice.” Oluronke thinks about Prince’s future but has learned to live in the moment and enjoy who her son is today.

On community:
“I know other children have been born with the same diagnosis. I want these parents to know I am here to help. If I got through the rough times, so will they.”
Oluronke, mom to Prince